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A 4-year-old boy with pulmonary capillaritis, treated with rituximab, steroids, and azathioprine for 2 years, presented with a 6-month history of falls and paucity of speech. Examination revealed irritability, mutism, hyperreflexia, and wide-based gait, and he had an Expanded Disability Status Scale (EDSS) score of 6.5. MRI brain revealed volume loss with extensive, bilateral T2/fluid-attenuated inversion recovery hyperintensities in the deep subcortical white matter, mesial temporal lobes, insula, globus pallidi, thalami, brainstem, and cerebellum (figure). MRI spine revealed increased T2 signal within the central dorsal aspect of the cervical cord. Lumbar puncture showed leukocyte count of 13 × 106/L (65% lymphocytes), erythrocyte count of 1 × 106/L, glucose 2.7 mmol/L, and protein 0.36 g/L. NMDAR antibody testing was negative (serum/CSF). Nasopharyngeal and bronchoalveolar lavage samples tested positive for enterovirus/rhinovirus by multiplex PCR and enterovirus was detected by RT-PCR (serum/CSF).
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Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received November 12, 2018.
- Accepted in final form February 12, 2019.
- © 2019 American Academy of Neurology
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